CR, a sixty-eight year old woman, was admitted via the ED with acute respiratory failure and aspiration pneumonia. CR had amyotrophic lateral sclerosis (ALS), an incurable, progressive, neuromuscular disease. According to Lewis, Heitkemper, and Dirksen, 2000, ALS is a fatal neuromuscular disease that attacks nerve cells and pathways in the brain and around the spinal cord. All voluntary muscle actions are affected and the person eventually becomes paralyzed. The mind, however, is not affected, so the person retains cognitive function despite the total degeneration of the body. Death usually results from respiratory infection secondary to compromised respiratory function. CR had experienced severe difficulty with swallowing, difficulty with oral secretions, and respiratory distress for several days before calling emergency services. CR's primary physician had not referred her to a hospice for palliation of end-stage symptoms of ALS, nor had he offered her palliative care. CR did not want "heroic measures", but she was in such severe distress at home that she believed she had no other options. She accepted emergency intubation and ventilation in the ED because she felt she was "suffocating."
CR was admitted to our critical care unit. On the next hospital day, CR wrote a note asking about her options. She wrote, "I don't want to be on machines." The ICU staff explained all her options to her, including permanent tracheostomy, home ventilation, and the use of an enteral feeding tube to provide nutrition. CR was also told that her pulmonary function parameters were so compromised that she was unlikely to survive ventilator withdrawal for more than several minutes, if she chose ventilator withdrawal.
I was caring for CR and she wrote me to call her sister, TJ, who was ...